Friconnet Guillaume, Duchesne Mathilde, Gueye Marcel, Caire François, Mounayer Charbel, Emile Jean-François, Labrousse François, Rouchaud Aymeric
Department of Radiology, Centre Hospitalier et Universitaire Dupuytren, Limoges, France.
Department of Pathology, Centre Hospitalier et Universitaire Dupuytren, Limoges, France.
Radiol Case Rep. 2021 Apr 30;16(7):1613-1617. doi: 10.1016/j.radcr.2021.04.021. eCollection 2021 Jul.
Rosai-Dorfman disease is a non-Langherans cell histiocytosis typically revealed by a lymphadenopathy. Central nervous system involvement is rare, exceptionally isolated, and usually consists of dural masses mimicking meningioma. Very few reports have described non-dural-based lesions, especially with an intra-ventricular development. We report hereby the case of a Rosai-Dorfman disease in a 30-year-old man presenting as an isolated mass arising from the right cerebellar peduncle and protruding into the fourth ventricle. We provide the results of the MRI examination with a special focus on advanced MRI features. As the diagnosis relies on pathological examination, we also detail the results of the analysis that followed the surgical resection of the mass including the immunohistochemical profile. This report highlights the necessity to consider Rosai-Dorfman disease as a potential diagnosis in case of an infra-tentorial mass and/or intra-ventricular mass.
罗萨伊-多夫曼病是一种非朗格汉斯细胞组织细胞增多症，通常表现为淋巴结病。中枢神经系统受累罕见，极为孤立，通常表现为类似脑膜瘤的硬脑膜肿块。很少有报告描述非硬脑膜性病变，尤其是脑室内部发展的病变。我们在此报告一例30岁男性的罗萨伊-多夫曼病，表现为起源于右侧小脑脚并突入第四脑室的孤立肿块。我们提供了MRI检查结果，特别关注了先进的MRI特征。由于诊断依赖于病理检查，我们还详细介绍了肿块手术切除后的分析结果，包括免疫组化特征。本报告强调，对于幕下肿块和/或脑室内肿块，有必要考虑罗萨伊-多夫曼病作为潜在诊断。
